title: [Acute neutrophilic dermatosis (pustular dermatitis) associated with aggressive transformed mycosis fungoides].
abstract: BACKGROUND
  Large-cell transformation in mycosis fungoides [MF] is associated with an aggressive clinical course and poor survival. In addition to tumour progression, infectious complications may be the cause of death. We report a case of aseptic pustular dermatosis in transformed MF that very quickly became aggressive and was followed by rapid death of the patient.
  
  
  METHODS
  A 47-year-old man presented with disseminated CD30+ transformed MF skin tumours refractory to chemotherapy for nine consecutive months. Ten days after a first course of dexamethasone, cytarabine and carboplatin, he was hospitalized for febrile neutropenia. Following this episode of neutropenia, he developed a febrile pustular rash disseminated on the tumours and on plaques, associated with marked neutrophilic leucocytosis. Bacteriological and mycological skin and blood samples were negative. Tumour biopsies showed a dermal infiltrate of CD30+ T-cells corresponding to the transformed MF, mixed with neutrophils. The pustule biopsies revealed intra-epidermal pustules and dermal-epidermal infiltration of neutrophils. The pustular eruption regressed with corticosteroids. Despite a last line of treatment with romidepsin, the patient died 6 weeks after the "neutrophilic" event.
  
  
  CONCLUSIONS
  Episodes of neutrophilic dermatosis [ND] are frequently seen in haematological disease and some substances can induce a variant form of eccrine hidradenitis. However, our patient's presentation was different, without the appearance of "classic" ND but rather diffuse pustular lesions carrying a poor prognosis, which was followed by rapid death. Once the hypothesis of infectious complications has been ruled out, treatment is based on systemic corticosteroids associated with specific treatment of the lymphoma.
authors.affiliation: Service de dermatologie, hôpital de Haut-Lévêque, CHU de Bordeaux, avenue de Magellan, 33600 Pessac, France.
authors.forename: S
authors.initials: S
authors.lastname: Guillet
authors.forename: J
authors.initials: J
authors.lastname: Stokkermans
authors.forename: B
authors.initials: B
authors.lastname: Vergier
authors.forename: M-S
authors.initials: MS
authors.lastname: Doutre
authors.forename: M
authors.initials: M
authors.lastname: Beylot-Barry
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000009:adverse effects; D047630:Depsipeptides / Q000627:therapeutic use; D018450:Disease Progression; D004317:Doxorubicin / Q000008:administration & dosage / Q000009:adverse effects; D019008:Drug Resistance, Neoplasm; D017809:Fatal Outcome; D064147:Febrile Neutropenia / Q000139:chemically induced / Q000150:complications / Q000188:drug therapy; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000150:complications* / Q000188:drug therapy; D009361:Neoplasm Invasiveness; D009504:Neutrophils / Q000473:pathology; D010198:Pancytopenia / Q000209:etiology; D011092:Polyethylene Glycols / Q000627:therapeutic use; D011241:Prednisone / Q000008:administration & dosage / Q000009:adverse effects; D016463:Sweet Syndrome / Q000209:etiology* / Q000473:pathology; D014750:Vincristine / Q000008:administration & dosage / Q000009:adverse effects
chemical_list: D000305:Adrenal Cortex Hormones; D047630:Depsipeptides; C486981:pegylated granulocyte colony-stimulating factor, human; D016179:Granulocyte Colony-Stimulating Factor; D011092:Polyethylene Glycols; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; C087123:romidepsin; D011241:Prednisone
keywords: Aseptic pustules; Dermatose neutrophilique; Mycosis fongoïde transformé; Neutrophilic dermatosis; Pustules aseptiques; Transformed mycosis fungoides